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Pityriasis lichenoides et varioliformis acuta in skin of color: new observations by dermoscopy Balachandra S. Ankad1, Savitha L. Beergouder1 1 Department of Dermatology, S. Nijalingappa Medical College, Bagalkot, Karnataka, India

Key words: dermoscopy, pityriasis lichenoides et varioliformis acuta, diagnosis, pattern Citation: Ankad BS, Beergouder SL. Pityriasis lichenoides et varioliformis acuta in skin of color: new observations by dermoscopy. Dermatol Pract Concept. 2017;7(1):5. DOI: http://dx.doi.org/10.5826/dpc.0701a05 Received: June 19, 2016; Accepted: October 28, 2016; Published: January 31, 2017 Copyright: ©2017 Ankad et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Funding: None. Competing interests: The authors have no conflicts of interest to disclose. All authors have contributed significantly to this publication. Corresponding author: Dr. Balachandra S. Ankad, Professor, Department of Dermatology, S. Nijalingappa Medical College, Near APMC, Navanagar, Bagalkot-587103, Karnataka, India. Tel. 91 9980410056; Fax: 08354 235360. E-mail: [email protected]

ABSTRACT

Background: Pityriasis lichenoides is an uncommon skin disease that presents in three different forms: pityriasis lichenoides et varioliformis acuta (PLEVA), pityriasis lichenoides chronica (PLC) and febrile ulceronecrotic-Mucha-Habermann disease. These represent a spectrum of a disease. PLEVA presents as skin eruption of multiple, small, red papules that develop into polymorphic lesions with periods of varying remissions, as well as possible sequels of hyper/hypopigmentation and varicella-like scars. Diagnosis of this condition is mainly clinical, and sometimes clinical differentiation from other conditions may be a difficult task that often requires histological analysis. In this study, PLEVA lesions were examined by dermoscopy, and the significance of specific dermoscopic findings was investigated in order to facilitate their differentiation from other inflammatory conditions. Objectives: To evaluate dermoscopic patterns in PLEVA and to correlate these patterns with histopathology. Materials and methods: The study was conducted at S. Nijalingappa Medical College, Bagalkot. It was an observational case series study and patients were selected randomly. Ethical clearance and informed consent were obtained. PLEVA lesions in early and late phases were evaluated. A manual DermLite 3 (3Gen, San Juan Capistrano, CA) dermoscope attached to a Sony (Cyber Shot DSC-W800, Sony Electronics Inc., San Diego, California, USA, digital, 14 mega pixels) camera was employed. Histopathology was done to confirm the diagnosis. Data was collected and analyzed. Results were statistically described in terms of frequencies and types of dermoscopic patterns. Results: There was a total of 14 patients; 8 males and 6 females. Mean age of patients was 19 years. Mean duration of disease was 7 months. Dermoscopy in early-phase lesions revealed amorphous brownish areas around the hair follicles, dotted vessels, and scaling. Dermoscopy in late-phase lesions showed whitish-structureless areas and central white crust within whitish-structureless rim with scale, focal bluish-grayish areas or centrifugal strands irregularly distributed along the periphery and yellow structures. Red dots and hemorrhage were seen at the center and glomerular vessels at the periphery. Conclusion: PLEVA demonstrates specific dermoscopic patterns that correlate well with histologic changes. New dermoscopic findings are described. Thus, dermoscopy is a good diagnostic tool in the clinical diagnosis of PLEVA.

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Introduction Pityriasis lichenoides (PL), also called Mucha-Habermann disease, is characterized by multiple crusted and scaly papules. It includes three variants, namely, pityriasis lichenoides et varioliformis acuta (PLEVA), pityriasis lichenoides chronica (PLC) and febrile ulceronecrotic PLEVA. PLEVA can mimic various skin conditions such as chicken pox, lymphomatoid papulosis, guttate psoriasis, lichen planus and pityriasis rosea [1,2]. Dermoscopy is a non-invasive technique that allows a

ultrasound gel was applied either on the faceplate of the dermoscope or on the skin lesion and then lesions were observed through the eyepiece of the dermoscope. However, only the polarized light version was used in our study to appreciate color patterns in the dermis. Although polarized dermoscopy was employed, ultrasound gel was applied for clarity of images and to lessen distortions associated with light [4]. Care was taken in holding the dermoscope a little above the surface of skin lesions to visualize the vascular structures [5].

rapid and magnified in vivo observation of the skin sur-

Inclusion criteria:

face. Dermoscopy is mainly utilized for the evaluation of

1. Patients with signs and symptoms of PLEVA [6],

pigmented skin lesions and has increasing applications in

2. Patients with PLEVA lesions in both early and late phases

dermatology [3]. Here, authors evaluated dermoscopic patterns in PLEVA in brown skin, and these patterns may assist the clinical diagnosis.

were included. 3. Patients who had not received or stopped treatment for PLEVA one month prior to the study.

Exclusion criteria:

Objectives

1. Patients with secondary infection superseding PLEVA. 2. Patients who were receiving treatment one month prior

To evaluate dermoscopic patterns in PLEVA and to correlate

to the study.

these patterns with histopathology.

Materials and Methods PATIENTS

Results There were 14 patients (8 male, 6 female) in the study. The patients with PLEVA with mean duration of disease of 7

This study was carried out on 14 patients attending the

months [minimum 2 months and maximum 12 months] and

Department of Dermatology in a tertiary hospital attached

mean age of 19 years presented with acute onset of erythema-

to S. Nijalingappa Medical College at Bagalkot, southern

tous papules with hemorrhagic crusting. Two (14.28%) and

India between March 2015 and December 2015. It was an

12 (85.71%) patients had early (Figure 1) and late (Figure 2)

observational case series study and patients were selected ran-

lesions of PLEVA respectively.

domly. A complete history and dermatological examination

Dermoscopy of early phase lesions: It showed amorphous

were performed on patients with clinical features suggestive

brownish area around the hair follicles and rim of scale in the

of PLEVA in early and late phases. The ethical clearance was

center and dotted vessels at periphery (Figure 3).

obtained by the institutional ethical committee. The patients

Dermoscopy of late phase lesions: It demonstrated whitish-

gave written informed consent. Demographic data, such as

structureless areas arranged in a haphazard pattern with vague

age, gender, and clinical variables in terms of site of lesions and disease duration were documented. Lesions with less than 2 months’ and more than 2 months’ duration were arbitrarily termed as early and late lesions. These were referred to as target lesions and were selected for dermoscopic examination. Skin biopsies were taken from target lesions to confirm the diagnosis. Same dermatologist evaluated dermoscopic patterns and was unaware of clinical diagnosis. The pathologist was also unaware of the diagnosis and same pathologist evaluated histopathological changes. Data was collected and analyzed. The results were statistically described as types of dermoscopic patterns.

DERMOSCOPIC EXAMINATION A DermLite 3 dermoscope (10x magnification) with both polarized and non-polarized lights was employed in the study. A Sony camera was attached to save the images. Initially,

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Figure 1. Clinical image of pityriasis lichenoides et varioliformis acuta with excoriated papules with crust. [Copyright: ©2017 Ankad et al.]

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Figure 4. Dermoscopy showed whitish structureless areas (black star) arranged in a haphazard pattern with vague glomerular (black circle) and dotted (yellow circle) vessels in the periphery. [Copyright: ©2017 Ankad et al.]

bluish-grayish areas or centrifugal strands irregularly distributed along the periphery, and yellow structures and red dots and hemorrhage within the central crust-plug (Figures 5, 6). Histopathological examination showed hyperkeratosis, Figure 2. Clinical image of pityriasis lichenoides et varioliformis acuta with erythematous papules and pustules with crust. [Copyright: ©2017 Ankad et al.]

epidermal ulceration with basophilic infiltration, vacuolar alteration of basal layer, necrotic keratinocytes, and pronounced lymphocytic infiltrate in the papillary dermis in wedge-shaped pattern along with perivascular infiltration in early phase (Figure 7). In late lesions, hyperkeratosis, acanthosis, erosion in the epidermis, basal cell degeneration and lymphocytic infiltrate around capillaries were seen (Figure 8) [7]. Dilatation of dermal capillaries was observed in both phases. Frequencies of various dermoscopic patterns are presented in Table 1, and corresponding histopathological changes are depicted in Table 2.

Figure 3. Dermoscopy pityriasis lichenoides et varioliformis acuta showing amorphous brownish area (yellow arrow) around the hair follicles and dotted vessels (black circle) and rim of scale. [Copyright: ©2017 Ankad et al.]

glomerular and dotted vessels in the periphery (Figure 4), central white crust and whitish-structureless rim with scale, focal

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Figure 5. Dermoscopy of pityriasis lichenoides et varioliformis acuta showing whitish-yellow structureless rim white scale (black circle), whitish crust in the centre (yellow arrow), focal bluish-grayish areas (black stars). Red dots and hemorrhage within the central crust-plug (black arrows). [Copyright: ©2017 Ankad et al.]

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Figure 6. Dermoscopy of pityriasis lichenoides et varioliformis acuta showing whitish-yellow structureless areas (black circle), focal bluish-grayish areas (yellow stars), hemorrhage (black arrow) and scale.

Figure 8. Histopathology in late phase shows hyperkeratosis, acanthosis, erosion in the epidermis, basal cell degeneration and lymphocytic infiltrate around capillaries with dilatation of blood vessels (H&E, 10 x). [Copyright: ©2017 Ankad et al.]

challenge to differentiate it from common conditions like chicken pox, guttate psoriasis, Gianotti-Crosti syndrome, lichen planus and pityriasis rosea, and in such situations histopathology helps to confirm the diagnosis [1]. Dermoscopy is a novel diagnostic technique; beside its traditional use in melanoma detection, it is being used more and more in the assessment of other general dermatologic conditions, namely scalp and hair disorders (trichoscopy), nails abnormalities (onychoscopy), skin infections and infestations (entomodermoscopy), and cutaneous inflammatory diseases (inflammoscopy) [3]. In this study, authors evaluated dermoscopic patterns in PLEVA. In this study, dermoscopy of early PLEVA lesions i.e., lesions aged less than 2 months revealed an amorphous Figure 7. Histopathology in early phase shows hyperkeratosis, epidermal ulceration with basophilic infiltration, vacuolar alteration of basal layer, necrotic keratinocytes and pronounced lymphocytic infiltrate in the papillary dermis in wedge shaped pattern along with perivascular infiltration and dilatation of dermal capillaries is seen (H&E, 10 x). [Copyright: ©2017 Ankad et al.]

brownish area around the hair follicles within a rim of white scale and dotted vessels at the periphery. In a study of dermoscopy of PLEVA by Lacarrubba et al, the authors observed an amorphous brownish structure and a ring of pinpoint and linear vessels in a “targetoid” pattern surrounding whitish-structureless areas [8]. Similar findings were observed in this study. However, the targetoid pattern

Discussion

of vessels was not observed. Vessels were in dotted pattern

PLEVA presents with a skin eruption of multiple, small, red

extravasations of red blood cells in the papillary dermis. This

papules that develop into polymorphic lesions and vacillates

disparity may be the result of the early stage of lesions and the

with periods of varying remissions as well as possible sequels

dermoscopic technique followed in this study. Interestingly,

of hyper/hypopigmentation and varicella-like scars. PLEVA

an amorphous brownish structure was noted around the hair

is mainly diagnosed clinically, but sometimes it poses a great

follicle. This finding was not mentioned in the previous study.

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at the periphery. These correspond to microhemorrhages and

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TABLE 1. Dermoscopic patterns and their frequency. [Copyright: ©2017 Ankad et al.] Frequency Sl. No.

Dermoscopic patterns (n=14), n (%)

01

02

White structures • Whitish-structureless areas in haphazard pattern • Whitish-structureless rim with central crust-plug Red structures • Dotted vessels • Hemorrhages • Vague glomerular

2 (14.28) 10 (85.71) 8 (57.14) 4 (28.57) 2 (14.28)

03

Focal bluish-grayish areas

10 (71.42)

04

Yellow globules or structures

10 (71.42)

05

Amorphous brownish areas

2 (14.28)

06

Scaling

8 (57.14)

TABLE 2. Dermoscopic patterns and corresponding histopathological changes. [Copyright: ©2017 Ankad et al.] Sl No.

Dermoscopic patterns

Corresponding histopathologic changes

01

Whitish-structureless areas

Hyperkeratosis and acanthosis

02

Whitish-structureless rim with central crust-plug

Hyperkeratosis and epidermal erosion

03

Red dots and hemorrhage

Microhemorrhages and extravasations of red blood cells in the papillary dermis and dilatation of blood vessels

04

Vague glomerular vessels

Dilatation of blood vessels

05

Focal bluish-grayish areas

Melanin pigment in the dermis

06

Yellow globules or structures

Spongiosis and basal cell degeneration

07

Amorphous brownish areas

Crust-plug with basophilic material in epidermis and lymphocytic infiltrate in the dermis

08

Scaling

Hyperkeratosis

An amorphous brownish structure corresponds to a central

supportive clue to the clinical diagnosis. In dermatofibroma,

crust consisting of basophilic material in the epidermis and

it takes on the “starburst” pattern [12], and in eccrine spi-

wedge-shaped lymphocytic infiltrate in the dermis.

radenoma, it is arranged as structureless areas surrounding

Late-phase lesions demonstrated whitish-structureless

serpentine vessels [13]. Histopathological correlation of white

areas and a central crust-plug surrounded by whitish rim

structures under dermoscopy depends on the condition. For

with scale. This is in correlation with previous observation

example, it suggests hyperkeratosis, acanthosis and dermal

[8]. Red dots and hemorrahges were observed in the cen-

fibrosis in hypertrophic lichen planus and prurigo nodularis;

ter, within the whitish rim. Whitish-structureless areas are

in morphea, eccrine spiradenoma and dermatofibroma it

seen in inflammatory conditions such as prurigo nodularis,

indicates increased collagen in the dermis. In PLEVA, it rep-

hypertrophic lichen planus, and morphea and in some of the

resents hyperkeratosis.Thus pattern of whitish-structureless

non-melanocytic skin tumors [9-13]. In hypertrophic lichen

areas gives a clue to the clinical diagnosis.

planus and prurigo nodularis it is referred to as pearly white

Focal bluish-grayish areas or strands distributed peripher-

structure spread over the entire lesion and seen characteristi-

ally were observed in late lesions. This finding is not reported

cally in a “starburst” pattern in prurigo nodularis; and in

in the previous study. The authors believe that this finding is

hypertrophic lichen planus it is distributed diffusely in the

due to scratching and skin of color. The configurations and

centre [9, 10]. In morphea, it appears as whitish strands with

patterns of focal bluish-grayish areas are specific for each

dilated blood vessels [11]. In non-melanocytic tumors, white

condition. They are discrete, ovoid nests pattern in pigmented

structures are arranged in a specific pattern, which give a

and superficial types of basal cell carcinoma [14]. In hyper-

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Figure 9. Image depicting correlation of dermoscopic patterns with histopathological changes in early phase of pityriasis lichenoides et varioliformis acuta. [Copyright: ©2017 Ankad et al.]

Figure 10. Image depicting correlation of dermoscopic patterns with histopathological changes in late phase of pityriasis lichenoides et varioliformis acuta. [Copyright: ©2017 Ankad et al.]

trophic lichen planus, they are distributed centrifugally within

Yellow structures were arranged diffusely within focal

pearly white areas [10]. In PLEVA, too, focal bluish-grayish

bluish-grayish areas and whitish structureless areas. These were

areas were seen interspersed within whitish-structureless

not described in PLEVA. However, orange-yellowish structure-

areas. Thus, arrangement of focal bluish-grayish areas is use-

less areas were observed in dermoscopic examination of pity-

ful clue to support the clinical diagnosis.

riasis lichenoides chronica by Errichetti et al. Orange-yellowish

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structureless areas correspond to hemosiderin deposition in the

hence require skin biopsy. Dermoscopy is a simple diagnostic

dermis [16]. In this study, the orange hue was absent and only

tool that helps in the diagnosis of PLEVA with specific and

the yellowish color was appreciated. Hence, the authors pro-

characteristic patterns that correlate well with histopatho-

pose that yellow structures in PLEVA are produced as a result

logical changes. New dermoscopic findings of PLEVA are

of the increased duration of lesions and that they represent

described here. However, this study was performed on a

spongiosis and basal cell degeneration on histology. Yellow

limited number of patients, and hence, further studies are

structures noted in hypertrophic lichen planus correspond to

warranted to elucidate these findings.

spongiosis and basal cell degeneration [10, 15]. In hypertrophic lichen planus, yellow structures are arranged in a “lacy network” pattern [10] and in dermatitis, they are in a diffuse pattern, giving rise to the “yellow clod” sign [17]. Red dots and hemorrhages represent focal hemorrhage and dilated vessels in the papillary dermis. The types of vessels and their arrangement form an important factor in the clinical diagnosis by dermoscopy. In this study, red dots and hemorrhage were situated in the center and vague glomerular and dotted vessels at the periphery. In the previous study of dermoscopy of PLEVA, the authors observed a ring of pinpoint and linear vessels in a “targetoid” pattern surrounding whitish-structureless areas [8]. In inflammatory conditions, vessels are distributed and arranged in a specific pattern, which aids in an accurate diagnosis. In psoriasis, red dots are diffuse in a regularly arranged pattern on an erythematous background. In pityriasis rosea, they are distributed in an irregular or patchy manner at the periphery on a yellowish base. The cluster pattern of vessels is characteristic in all types of dermatitis [17]. In seborrheic keratosis and clear cell carcinoma, vessels are in “hair-pin” and “string of pearls” patterns respectively [18]. In pityriasis lichenoides chronica, milky red areas/globules, linear irregular and branching vessels are seen [16]. Hence, the pattern of blood vessels is of diagnostic importance in the dermoscopic studies. In the present study, the authors noted focal bluishgrayish areas and yellow structures, which are not described in literature. The authors believe that the duration of lesions, scratching, and skin of color attribute to focal bluish-grayish areas and yellow structures. PLEVA lesions in the early phase showed an amorphous brownish area that correlated with basophilic material and wedge-shaped lymphocytic infiltrate on histology (Figure 9), whereas late-phase lesions demonstrated whitish structureless areas, focal bluish-grayish areas, and yellow structures corresponding to epidermal erosion, hyperkeratosis, spongiosis, basal cell degeneration in infiltrate on histology (Figure 10). However, limitations in this method do exist, and they include usage of ultrasound gel in polarized version of dermoscopy, the small study sample, and in that the same dermatologist performed dermoscopic evaluation all PLEVA lesions.

Conclusion PLEVA is a rare disorder that can be challenging to differentiate clinically from a number of common dermatoses, and

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Acknowledgements: The authors wish to acknowledge the help of Dr. Vijay Domble for his assistance with histopathology.

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